Ependymoma

Ependymoma is a tumor arising from the cells lining the ventricles and central canal of the spinal cord. It can occur in children or adults, at any level of the CNS, and its behavior varies dramatically based on where it is and what the molecular testing shows.

What Is Ependymoma?

Ependymomas are tumors of the ependymal cells lining the brain's ventricles and spinal cord canal. Spinal ependymomas are the most common primary spinal cord tumor in adults; intracranial ependymomas are the third most common brain tumor in children. The 2021 WHO classification defines multiple molecular subgroups with dramatically different behaviors.

Surgery is the cornerstone of treatment: complete resection is the single most powerful factor driving long-term control. The value of adjuvant radiation depends heavily on the molecular subgroup.

At a Glance

Ependymoma arises from cells lining the ventricles or spinal cord canal — affects both children and adults
The most common primary spinal cord tumor in adults; the third most common brain tumor in children
Gross total resection is the single most powerful factor driving long-term control
2021 WHO classification now defines molecular subgroups that predict behavior more accurately than grade alone
Spinal ependymomas: ~80-90% 5-year survival after GTR; intracranial: varies by molecular subgroup

Symptoms

Intracranial ependymoma (often in posterior fossa in children)

Headaches, nausea, vomiting (from hydrocephalus blocking CSF flow)
Unsteady gait, coordination problems
Cranial nerve palsies (diplopia, facial weakness) if brainstem adjacent

Spinal ependymoma (most common in adults)

Gradually progressive back or neck pain
Weakness, numbness or tingling in the arms or legs
Bladder and bowel dysfunction (especially with conus/cauda location)

Diagnosis

MRI with contrast: shows a well-circumscribed enhancing mass; spinal ependymomas often have a 'cap sign' (hemosiderin from chronic bleeding)

MRI of entire neuraxis: ependymoma can drop-metastasize through the CSF; full staging required

CSF cytology: lumbar puncture at least 2-3 weeks after surgery

Molecular testing: required for WHO 2021 classification: DNA methylation profiling, FISH for 1q25 (high-risk in posterior fossa), RELA/YAP1 fusion status

Types

Spinal ependymoma, NOS (most common adult spinal tumor)

Myxopapillary ependymoma (WHO grade 2) — conus/filum terminale; generally favorable

Subependymoma (WHO grade 1) — slow-growing, often incidental

Intracranial ependymoma

PFA (posterior fossa group A) — children; aggressive; 1q25 gain a bad prognostic marker
PFB (posterior fossa group B) — adolescents/adults; favorable
RELA-fusion or YAP1-fusion — supratentorial; RELA has aggressive behavior

Treatment

Maximal safe resection — goal is gross total resection for all types

In spinal ependymomas, a tissue plane between tumor and cord often permits complete removal — one of the only spinal cord tumors where GTR is regularly achievable. Use of intraoperative motor and somatosensory evoked potentials is standard.

Adjuvant radiation

For intracranial ependymoma: local conformal radiotherapy (54–59.4 Gy) recommended after GTR for PFA, grade 3, and recurrent disease. For completely resected low-grade spinal ependymoma, observation after GTR is acceptable.

For recurrence

Re-resection is always the first consideration. Radiosurgery for small recurrences. Second-line chemotherapy has limited efficacy.

Outcomes

Subtype	5-year PFS (GTR)	10-year OS	Notes
Spinal ependymoma, NOS	~80%	~90%	GTR achievable in majority; excellent prognosis
Myxopapillary ependymoma	~75%	~85%	Recurrence possible even after GTR — surveillance essential
Posterior fossa type B (PFB)	~70%	~85%	Favorable; older patients
Posterior fossa type A (PFA)	~30-40%	~50-70%	Most aggressive; 1q gain lowers survival further
RELA-fusion, supratentorial	~55-60%	~65-70%	Intensive local therapy; clinical trials available

References

Louis DN, et al. The 2021 WHO Classification of Tumors of the Central Nervous System. Neuro-Oncology. 2021;23:1231-1251. PMID: 34185076
Merchant TE. Current clinical challenges in childhood ependymoma: a focused update. Semin Radiat Oncol. 2014;24:227-232. PMID: 24969398
Mack SC, et al. Epigenomic alterations define lethal CIMP-positive ependymomas. Nature. 2014;506:445-450. PMID: 24554663

Our Specialists

Arthur DiPatri, MD

Pediatric Neurosurgery

Bakhtiar Yamini, MD

Neurosurgery

Peter Warnke, MD

Neurosurgery

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